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Describes a specific familial cardiomyopathy phenotype characterized by ventricular arrhythmias, repolarization abnormalities, and left ventricular hypertrophy with autosomal dominant inheritance not linked to the HLA region.
In familial cardiomyopathy (CM), different forms of myocardial abnormalities including asymmetric and symmetric hypertrophy and dilated left ventricles are presented, mostly showing varying hereditary penetrance. This study presents a family with CM including three major clinical manifestations: severe ventricular arrhythmias, repolarization abnormalities and left ventricular hypertrophy. This triad was strikingly consistent in the two generations examined. The familial pattern with an autosomal dominant inheritance did not show any linkage to the HLA region.
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Lindvall et al. (1983) studied this question.
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